CD56-negative extranodal nasal type NK/T-cell lymphoma

Department of Pathology, University of Utah, Salt Lake City, Utah 84112, USA.
Pediatric Blood & Cancer (Impact Factor: 2.39). 07/2010; 55(1):186-9. DOI: 10.1002/pbc.22489
Source: PubMed


Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare lymphoma that occurs predominantly in Asian adults. In this report, we describe the clinical and pathologic features of an unusual aggressive lymphoid neoplasm in a child and review the literature on NK/T-cell lymphoma in children. The patient was a 4-year-old Native American male with facial swelling, lymphadenopathy, and fevers. Biopsy demonstrated neoplastic lymphoid cells that expressed CD3, CD8, TIA-1, and EBV-encoded RNA without CD56. The patient failed multiagent chemotherapy and died of therapy-related complications. This case represents an extranodal NK/T-cell lymphoma, nasal type, with an unusual lack of CD56.

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    • "However, considering the findings of TIA-1+, EBER1+ and no TCR rearrangement, we finally diagnosed this lymphoma as extranodal NK/T-cell lymphoma (nasal type). Recently, Miles et al. reported a case of CD56-negative extranodal NK/T-cell lymphoma [15]. In that case, neoplastic lymphoid cells expressed CD3, TIA-1 and EBER1 with an unusual lack of CD56. "
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